DOCSLIB.ORG

Co-Existence of Relapsing Polychondritis and Crohn Disease

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Co-Existence of Relapsing Polychondritis and Crohn Disease Case report eISSN 2384-0293 Yeungnam Univ J Med 2021;38(1):70-73 https://doi.org/10.12701/yujm.2020.00304 Co-existence of relapsing polychondritis and Crohn disease treated successfully with infliximab Hye-In Jung1, Hyun Jung Kim1, Ji-Min Kim2, Ju Yup Lee1, Kyung Sik Park1, Kwang Bum Cho1, Yoo Jin Lee1 1Division of Gastroenterology, Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea 2Division of Rheumatology, Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea Received: April 28, 2020 Revised: May 16, 2020 Relapsing polychondritis (RP) is a rare, progressive immune-mediated systemic inflammatory dis- Accepted: May 25, 2020 ease of unknown etiology, characterized by recurrent inflammation of cartilaginous structures. Approximately 30% of RP cases are associated with other autoimmune diseases. However, the Corresponding author: co-occurrence of RP and Crohn disease (CD) has rarely been reported. Herein, we present a Yoo Jin Lee 35-year-old woman diagnosed with RP and CD, who was refractory to initial conventional medi- Department of Internal Medicine, cations, including azathioprine and glucocorticoid, but who subsequently responded to infliximab Keimyung University School of (IFX). For both diseases, remission was sustained with IFX. There has been no previous report re- Medicine, 1035 Dalgubeol-daero, garding the successful treatment of co-existing RP and CD with IFX. Dalseo-gu, Daegu 42601, Korea Tel: +82-53-258-7739 Keywords: Crohn disease; Inflammatory bowel diseases; Infliximab; Relapsing polychondritis Fax: +82-53-258-4341 E-mail: [email protected] Introduction 05-064). The patient provided written informed consent for pub- lication of clinical details and images. Relapsing polychondritis (RP) is a rare, immune-mediated A 35-year-old woman with polyarthralgia and intermittent fever multi-systemic disease characterized by inflammation of multiple for a 2-month duration was referred to our rheumatology clinic. cartilaginous tissues, particularly those in the ear, nose, tracheo- She presented with pain in multiple joints, including both hands, bronchial tree, and eye [1,2]. Although the pathogenesis of RP re- both wrists, right ankle, and both knees, and reported that the mains unclear, an immunological mechanism is considered to be pain was most prominent in both metacarpophalangeal and prox- crucial [1]. Accordingly, nearly 30% of RP reportedly coexists with imal interphalangeal joints. The patient was not on any medica- other autoimmune inflammatory disorders, including rheumatoid tion and had no prior illness. Laboratory tests revealed that C-re- arthritis, systemic lupus erythematosus, and Sjogren’s syndrome active protein was elevated to 6.32 mg/dL (range, 0–0.5 mg/dL), [3]. However, the co-occurrence of RP and Crohn disease (CD) is erythrocyte sedimentation rate was increased to 120 mm/hr rare, and the treatment strategy for such cases remains uncertain. (range, women < 25 mm/hr), hemoglobin was decreased to 10.4 Herein, we present a first report of co-existing RP and CD success- g/dL (range, 11–15 g/dL). Her rheumatoid factor was marginally fully treated with infliximab (IFX). elevated to 25.2 IU/mL (range, ≤ 20 IU/mL); however, anti-cy- clic citrullinated peptide antibody and antinuclear antibody were Case absent. All other biochemical parameters, leukocyte count, and electrolyte values were within normal limits. X-rays of multiple This study was approved by the Institutional Review Board of the joints were normal without evidence of erosion. Keimyung University Dongsan Hospital (IRB No: DSMC 2020- On physical examination, she presented bilateral injected con- Copyright © 2021 Yeungnam University College of Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 70 https://doi.org/10.12701/yujm.2020.00304 Yeungnam Univ J Med 2021;38(1):70-73 junctiva (Fig. 1), as well as bilateral auricular redness and tender- the terminal ileum, cecum, and ascending colon, favoring a diag- ness, with sparing of the earlobes (Fig. 2). Ophthalmological ex- nosis of CD. Brownish, discolored colonic mucosa was observed, amination revealed bilateral episcleritis. An auricular biopsy was which was considered melanosis coli associated with the intake of performed, and various inflammatory cells were observed in the aloe vera (Fig. 4). Histological analyses of the colonoscopic biop- chondro-dermal junction (Fig. 3). Based on auricular cartilage in- sy revealed chronic inflammation. Her calprotectin level was flammation, ocular inflammation, and seronegative arthritis, she > 1,000 μg/g (range, < 50 μg/g). Collectively, the patient was fi- was diagnosed with RP according to the RP diagnostic criteria nally diagnosed with RP co-existing with CD. documented by Michet et al. [4]. After discontinuing NSAID therapy, she was treated with me- Initial treatment was started with a low-dose steroid (predniso- salazine (4 g/day), azathioprine (50 mg/day), and intravenous lone 10 mg) and non-steroidal anti-inflammatory drugs (IV) high-dose corticosteroid. With high-dose corticosteroid (NSAIDs) at the outpatient clinic. Three months later, she visited treatment, both CD- and RP-related symptoms were markedly the emergency department with fever, abdominal pain, and inter- improved. However, after tapering steroid treatment, she com- mittent hematochezia occurring for a month. A detailed interview plained of mild, fluctuating, but persisting abdominal pain, diar- focusing on gastrointestinal manifestations revealed that she had rhea, bilateral auricular pain, and polyarthritis. Despite mainte- been experiencing intermittent vague abdominal pain and diar- nance therapy comprising azathioprine and repeated steroid res- rhea 3–4 times per week for one year. She had self-diagnosed her cue therapy, flare-ups occurred twice in 6 months; thus, IV IFX (5 symptoms as irritable bowel syndrome and had often consumed mg/kg) was initiated. Following induction therapy with IFX, all aloe vera as a folk remedy. Interestingly, she reported that her ab- symptoms, including abdominal pain, diarrhea, auricular pain, dominal pain had repeatedly worsened, several times along with and arthralgia, were improved. Eighteen months after treatment the exacerbation of RP-related symptoms, such as arthralgia, con- with IFX, the colonoscopy showed that only ulcer scars and a few junctival injection, and bilateral auricular tenderness. Colonosco- inflammatory pseudopolyps remained. She had no RP recurrence py revealed multiple discontinuous ulcers with skipped lesions in and was confirmed to have reached not only a clinical but also an endoscopic remission with IFX. Discussion RP is a long-lasting, systemic inflammatory disease presenting di- verse clinical manifestations, with a flaring‒remitting course [3]. Fig. 1. Photo of patient's eye. Bilateral conjunctival injection is observed. Ophthalmologic examination presents corneal ulcer and iritis. A B Fig. 3. Microscopic finding of auricular biopsy. The microscopic Fig. 2. Photos of both ears. The bilateral erythema of both finding of an auricular lesion shows perivascular infiltration auricles with sparing of the ear lobes (A, right; B, left) indicates of lymphocytes in a background of fibrous connective tissue bilateral auricular chondritis. (hematoxylin and eosin stain, ×200). https://doi.org/10.12701/yujm.2020.00304 71 Jung HI et al. Co-existence of relapsing polychondritis and Crohn disease both wrists, right ankle, and both knees, with episcleritis and unique auricular chondritis appeared simultaneously, which con- curred with the criteria (B) of Michet et al. [4]. Although the etiology of RP remains unclear, an autoimmune RP hypothesis is supported owing to its frequent association with other autoimmune diseases, as well as the effectiveness of cortico- steroid and immunosuppressant treatments [2]. As both RP and A B IBD are immune-mediated diseases, they can theoretically appear together. Reportedly, RP has been introduced as one of the ex- tra-intestinal manifestations (EIMs) of IBD, but compared to oth- er EIMs, such as peripheral and axial arthropathies, erythema no- dosum, and pyoderma gangrenosum, RP is an extremely rare EIM of IBD [8,9]. Furthermore, RP is easily overlooked by clinicians, owing to its non-specific and unusual manifestations [2]. Howev- er, proper diagnosis and treatment of RP are essential, as it can C D cause severe functional complications, including deafness, loss of vision, balance disturbance, and airway obstruction [3,10]. Fig. 4. Colonoscopic findings. Multiple discontinuous ulcers are Owing to a lack of data, standardized RP treatment guidelines are present in the terminal ileum (A, B), cecum, and ascending colon yet to be established. Currently, the treatment strategy remains em- (C, D). Melanosis coli is observed probably due to the previous pirical, based on existing case reports. The treatment goal is symp- history of aloe vera consumption (C, D). tomatic control and the long-term inhibition of immune-mediated pathogenic mechanisms [1]. Thus, empirical tailored therapy, ac- cording to the severity of disease activity and organ involvement, As RP is an extremely rare disease, with an estimated prevalence has been provided [10]. Mild forms of RP with the involvement
Load more

Recommended publications
  • Severe Septicaemia in a Patient with Polychondritis and Sweet's
    81 LETTERS Ann Rheum Dis: first published as 10.1136/ard.62.1.88 on 1 January 2003. Downloaded from Severe septicaemia in a patient with polychondritis and Sweet’s syndrome after initiation of treatment with infliximab F G Matzkies, B Manger, M Schmitt-Haendle, T Nagel, H-G Kraetsch, J R Kalden, H Schulze-Koops ............................................................................................................................. Ann Rheum Dis 2003;62:81–82 D Sweet first described an acute febrile neutrophilic dermatosis in 1964 characterised by acute onset, fever, Rleucocytosis, and erythematous plaques.1 Skin biopsy specimens show infiltrates consisting of mononuclear cells and neutrophils with leucocytoclasis, but without signs of vasculi- tis. Sweet’s syndrome is frequently associated with solid malig- nancies or haemoproliferative disorders, but associations with chronic autoimmune connective tissue disorders have also been reported.2 The aetiology of Sweet’s syndrome is unknown, but evidence suggests that an immunological reaction of unknown specificity is the underlying mechanism. CASE REPORT A 51 year old white man with relapsing polychondritis (first diagnosed in 1997) was admitted to our hospital in June 2001 with a five week history of general malaise, fever, recurrent arthritis, and complaints of morning stiffness. Besides Figure 1 autoimmune polychondritis, he had insulin dependent Manifestation of Sweet’s syndrome in a patient with relapsing polychondritis. diabetes mellitus that was diagnosed in 1989. On admission, he presented with multiple small to medium, sharply demarked, raised erythematous plaques on both fore- dose of glucocorticoids (80 mg) and a second application of http://ard.bmj.com/ arms and lower legs, multiple acne-like pustules on the face, infliximab (3 mg/kg body weight) were given.
    [Show full text]
  • A Resident's Guide to Pediatric Rheumatology
    A RESIDENT’S GUIDE TO PEDIATRIC RHEUMATOLOGY 4th Revised Edition - 2019 A RESIDENT’S GUIDE TO PEDIATRIC RHEUMATOLOGY This guide is intended to provide a brief introduction to basic topics in pediatric rheumatology. Each topic is accompanied by at least one up-to-date reference that will allow you to explore the topic in greater depth. In addition, a list of several excellent textbooks and other resources for you to use to expand your knowledge is found in the Appendix. We are interested in your feedback on the guide! If you have comments or questions, please feel free to contact us via email at [email protected]. Supervising Editors: Dr. Ronald M. Laxer, SickKids Hospital, University of Toronto Dr. Tania Cellucci, McMaster Children’s Hospital, McMaster University Dr. Evelyn Rozenblyum, St. Michael’s Hospital, University of Toronto Section Editors: Dr. Michelle Batthish, McMaster Children’s Hospital, McMaster University Dr. Roberta Berard, Children’s Hospital – London Health Sciences Centre, Western University Dr. Liane Heale, McMaster Children’s Hospital, McMaster University Dr. Clare Hutchinson, North York General Hospital, University of Toronto Dr. Mehul Jariwala, Royal University Hospital, University of Saskatchewan Dr. Lillian Lim, Stollery Children’s Hospital, University of Alberta Dr. Nadia Luca, Alberta Children’s Hospital, University of Calgary Dr. Dax Rumsey, Stollery Children’s Hospital, University of Alberta Dr. Gordon Soon, North York General Hospital and SickKids Hospital Northern Clinic in Sudbury, University
    [Show full text]
  • Immunopathologic Studies in Relapsing Polychondritis
    Immunopathologic Studies in Relapsing Polychondritis Jerome H. Herman, Marie V. Dennis J Clin Invest. 1973;52(3):549-558. https://doi.org/10.1172/JCI107215. Research Article Serial studies have been performed on three patients with relapsing polychondritis in an attempt to define a potential immunopathologic role for degradation constituents of cartilage in the causation and/or perpetuation of the inflammation observed. Crude proteoglycan preparations derived by disruptive and differential centrifugation techniques from human costal cartilage, intact chondrocytes grown as monolayers, their homogenates and products of synthesis provided antigenic material for investigation. Circulating antibody to such antigens could not be detected by immunodiffusion, hemagglutination, immunofluorescence or complement mediated chondrocyte cytotoxicity as assessed by 51Cr release. Similarly, radiolabeled incorporation studies attempting to detect de novo synthesis of such antibody by circulating peripheral blood lymphocytes as assessed by radioimmunodiffusion, immune absorption to neuraminidase treated and untreated chondrocytes and immune coprecipitation were negative. Delayed hypersensitivity to cartilage constituents was studied by peripheral lymphocyte transformation employing [3H]thymidine incorporation and the release of macrophage aggregation factor. Positive results were obtained which correlated with periods of overt disease activity. Similar results were observed in patients with classical rheumatoid arthritis manifesting destructive articular changes. This study suggests that cartilage antigenic components may facilitate perpetuation of cartilage inflammation by cellular immune mechanisms. Find the latest version: https://jci.me/107215/pdf Immunopathologic Studies in Relapsing Polychondritis JERoME H. HERmAN and MARIE V. DENNIS From the Division of Immunology, Department of Internal Medicine, University of Cincinnati Medical Center, Cincinnati, Ohio 45229 A B S T R A C T Serial studies have been performed on as hematologic and serologic disturbances.
    [Show full text]
  • Relapsing Polychondritis
    Relapsing polychondritis Author: Professor Alexandros A. Drosos1 Creation Date: November 2001 Update: October 2004 Scientific Editor: Professor Haralampos M. Moutsopoulos 1Department of Internal Medicine, Section of Rheumatology, Medical School, University of Ioannina, 451 10 Ioannina, GREECE. [email protected] Abstract Keywords Disease name and synonyms Diagnostic criteria / Definition Differential diagnosis Prevalence Laboratory findings Prognosis Management Etiology Genetic findings Diagnostic methods Genetic counseling Unresolved questions References Abstract Relapsing polychondritis (RP) is a multisystem inflammatory disease of unknown etiology affecting the cartilage. It is characterized by recurrent episodes of inflammation affecting the cartilaginous structures, resulting in tissue damage and tissue destruction. All types of cartilage may be involved. Chondritis of auricular, nasal, tracheal cartilage predominates in this disease, suggesting response to tissue-specific antigens such as collagen II and cartilage matrix protein (matrillin-1). The patients present with a wide spectrum of clinical symptoms and signs that often raise major diagnostic dilemmas. In about one third of patients, RP is associated with vasculitis and autoimmune rheumatic diseases. The most commonly reported types of vasculitis range from isolated cutaneous leucocytoclastic vasculitis to systemic polyangiitis. Vessels of all sizes may be affected and large-vessel vasculitis is a well-recognized and potentially fatal complication. The second most commonly associated disorder is autoimmune rheumatic diseases mainly rheumatoid arthritis and systemic lupus erythematosus . Other disorders associated with RP are hematological malignant diseases, gastrointestinal disorders, endocrine diseases and others. Relapsing polychondritis is generally a progressive disease. The majority of the patients experience intermittent or fluctuant inflammatory manifestations. In Rochester (Minnesota), the estimated annual incidence rate was 3.5/million.
    [Show full text]
  • Pharmacy Policy Statement
    PHARMACY POLICY STATEMENT Ohio Medicaid DRUG NAME Actemra (tocilizumab) BILLING CODE For medical - J3262 (1 unit = 1 mg) For Rx - must use valid NDC BENEFIT TYPE Medical or Pharmacy SITE OF SERVICE ALLOWED Outpatient/Office/Home COVERAGE REQUIREMENTS Prior Authorization Required (Preferred Product) QUANTITY LIMIT— 3200 units per 28 days LIST OF DIAGNOSES CONSIDERED NOT Click Here MEDICALLY NECESSARY Actemra (tocilizumab) is a preferred product and will only be considered for coverage under the medical or pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. GIANT CELL ARTERITIS (GCA) For initial authorization: 1. Member must be 50 years of age or older; AND 2. Medication must be prescribed by a rheumatologist; AND 3. Must have a documented negative TB test (i.e., tuberculosis skin test (PPD), an interferon-release assay (IGRA)) within 12 months prior to starting therapy; AND 4. Member has a history of erythrocyte sedimentation rate (ESR) ≥ 50 mm/h or history of C - reactive protein (CRP) ≥ 2.45 mg/dL documented in chart notes OR if member received glucocorticoid (prednisone) therapy ESR ≥ 30 mm/h and CRP ≥ 1 mg/dL; AND 5. At least one of the following: a) Unequivocal cranial symptoms of GCA (new onset localized headache, scalp or temporal artery tenderness, ischemia-related vision loss, or otherwise unexplained mouth or jaw pain upon mastication); b) Unequivocal symptoms of polymyalgia rheumatica (PMR), defined as shoulder and/or hip girdle pain associated with inflammatory stiffness; AND 6. At least one of the following: a) Temporal artery biopsy revealing features of GCA; b) Evidence of large-vessel vasculitis by angiography; c) Cross-sectional imaging (such as MRI, CTA or PET-CT); AND 7.
    [Show full text]
  • 21362 Arthritis Australia a to Z List
    ARTHRITISINFORMATION SHEET Here is the A to Z of arthritis! A D Goodpasture’s syndrome Achilles tendonitis Degenerative joint disease Gout Achondroplasia Dermatomyositis Granulomatous arteritis Acromegalic arthropathy Diabetic finger sclerosis Adhesive capsulitis Diffuse idiopathic skeletal H Adult onset Still’s disease hyperostosis (DISH) Hemarthrosis Ankylosing spondylitis Discitis Hemochromatosis Anserine bursitis Discoid lupus erythematosus Henoch-Schonlein purpura Avascular necrosis Drug-induced lupus Hepatitis B surface antigen disease Duchenne’s muscular dystrophy Hip dysplasia B Dupuytren’s contracture Hurler syndrome Behcet’s syndrome Hypermobility syndrome Bicipital tendonitis E Hypersensitivity vasculitis Blount’s disease Ehlers-Danlos syndrome Hypertrophic osteoarthropathy Brucellar spondylitis Enteropathic arthritis Bursitis Epicondylitis I Erosive inflammatory osteoarthritis Immune complex disease C Exercise-induced compartment Impingement syndrome Calcaneal bursitis syndrome Calcium pyrophosphate dehydrate J (CPPD) F Jaccoud’s arthropathy Crystal deposition disease Fabry’s disease Juvenile ankylosing spondylitis Caplan’s syndrome Familial Mediterranean fever Juvenile dermatomyositis Carpal tunnel syndrome Farber’s lipogranulomatosis Juvenile rheumatoid arthritis Chondrocalcinosis Felty’s syndrome Chondromalacia patellae Fibromyalgia K Chronic synovitis Fifth’s disease Kawasaki disease Chronic recurrent multifocal Flat feet Kienbock’s disease osteomyelitis Foreign body synovitis Churg-Strauss syndrome Freiberg’s disease
    [Show full text]
  • PRES Abstracts 1-99
    17th Pediatric Rheumatology European Society Congress September 9-12, 2010 València, Spain Abstracts Page no. Oral Abstracts (1 – 36) xxx Poster Abstracts (1 – 306) xxx Clinical and Experimental Rheumatology 2011; xx: xxx-xxx. Oral abstracts 17th Pediatric Rheumatology European Society Congress Oral Abstracts RESULTS: This study had >80% to detect an odds ratio >1.25 for SNPs with allele frequencies >0.1. Two SNPs in the MVK gene, rs1183616 (ptrend=0.006 OR 1.17 95% CI 1.04-1.30) and rs7957619 (ptrend=0.005 OR 1.23 95% CI 1.07- O 01 1.43) are significantly associated with JIA. These two SNPs are in modest linkage Distinctive gene expression in patients with juvenile spondylo- disequilibrium (r2=0.36, D’=1). Logistic regression of the two SNPs, after condi- arthropathy is related to autoinflammatory diseases tioning on the most significant SNP, found that the rs1183616 SNP was no longer significant (p=0.3), suggesting that the association is a single effect driven by the Marina Frleta, Lovro Lamot, Fran Borovecki, Lana Tambic Bukovac, Miroslav rs7957619 SNP. This SNP lies within exon 3 of the MVK gene and is a Serine to Harjacek Asparagine substitution at position 52. There was no significant evidence of a dif- Children’s Hospital Srebrnjak, Srebrnjak, Zagreb, Croatia ference in allele frequencies between the seven ILAR subtypes for the rs7957619 SNP (p=0.32). INTRODUCTION: Juvenile Spondyloarthropathies (jSpA) are characterized by One SNP at the 3’ end of the TNFRSF1A gene, which actually lies within the dysregulation of the inflammatory processes and bone metabolism which may be adjacent gene SLCNN1A, rs2228576, was associated with protection from JIA clarified by gene expression profiles.
    [Show full text]
  • View a Copy of This Licence, Visit Iveco​ Mmons.​ Org/​ Licen​ Ses/​ By/4.​ 0/​
    Leyens et al. Orphanet J Rare Dis (2021) 16:326 https://doi.org/10.1186/s13023-021-01945-8 RESEARCH Open Access The combined prevalence of classifed rare rheumatic diseases is almost double that of ankylosing spondylitis Judith Leyens1,2, Tim Th. A. Bender1,3, Martin Mücke1, Christiane Stieber4, Dmitrij Kravchenko1,5, Christian Dernbach6 and Matthias F. Seidel7* Abstract Background: Rare diseases (RDs) afect less than 5/10,000 people in Europe and fewer than 200,000 individuals in the United States. In rheumatology, RDs are heterogeneous and lack systemic classifcation. Clinical courses involve a variety of diverse symptoms, and patients may be misdiagnosed and not receive appropriate treatment. The objec- tive of this study was to identify and classify some of the most important RDs in rheumatology. We also attempted to determine their combined prevalence to more precisely defne this area of rheumatology and increase awareness of RDs in healthcare systems. We conducted a comprehensive literature search and analyzed each disease for the speci- fed criteria, such as clinical symptoms, treatment regimens, prognoses, and point prevalences. If no epidemiological data were available, we estimated the prevalence as 1/1,000,000. The total point prevalence for all RDs in rheumatol- ogy was estimated as the sum of the individually determined prevalences. Results: A total of 76 syndromes and diseases were identifed, including vasculitis/vasculopathy (n 15), arthritis/ arthropathy (n 11), autoinfammatory syndromes (n 11), myositis (n 9), bone disorders (n 11),= connective tissue diseases =(n 8), overgrowth syndromes (n 3), =and others (n 8).= Out of the 76 diseases,= 61 (80%) are clas- sifed as chronic, with= a remitting-relapsing course= in 27 cases (35%)= upon adequate treatment.
    [Show full text]
  • Relapsing Polychondritis
    Disclosure statement Relapsing Collaborative research agreement with Polychondritis Novo Nordisk Jane Hoyt Buckner, MD Benaroya Research Institute Virginia Mason Medical Center Seattle WA 11/6/2011 Jane Hoyt Buckner 1 Topics of Discussion What is RP? Relapsing Polychondritis is an inflammatory disease of the hyaline cartilage Spectrum of disease in RP Hallmarks of the disease- inflammation of cartilage Diagnosis involving: Pathogenic mechanisms of disease • Auricular • Management Nasal • Costochondral Case Studies • Respiratory Tract • Joints Lahmer et al Autoimmunity Reviews (2010) 540–546 What is RP? Beyond Chrondritis Diagnostic Criteria Inflammation of structural component of end Clinical Manifestations organs: McAdam’s Criteria Bilateral auriclar chondritis require: 3 clinical Nonerosive, seronegative inflammatory polyarthritis manifestations, Nasal chondritis • Ocular with biopsy Ocular inflammation • Vasculopathy confirmation unless the Respiratory tract chondritis • Vasculitis diagnosis is Cochlear and /or vestibular dysfunction • Nephritis clinically obvious. Pathology • Encephalitis Cartilage biopsy with histologic findings compatible with relapsing polychondritis. Ref. Medicine 55:193, 1976 Lahmer et al Autoimmunity Reviews (2010) 540–546 Relapsing Polychondritis 1 Epidemiology Auricular Chondritis Frequency in the population Over 550 cases reported Inflammation of the external ear is the most common manifestation of 3.5 cases/million (Rochester, Minnesota) RP Age 83% of patients mean at onset 40-50 Erythema, tenderness and swelling Ranging from 16-90 present for days-weeks Gender Sparing of the ear lobe is typically F:M=1:1 found BRI RP registry Thickening and or drooping of ear 14 patients/3 million King County lobe may result from a flare of RP. 178 patients participating from the United States. Auricular Chondritis Clinical Manifestations Audiovestibular Symptoms: Differential diagnosis • Cellulitis .
    [Show full text]
  • Rheumatology
    THE AMERICAN BOARD OF PEDIATRICS® CONTENT OUTLINE Pediatric Rheumatology Subspecialty In-Training, Certification, and Maintenance of Certification (MOC) Examinations INTRODUCTION This document was prepared by the American Board of Pediatrics Subboard of Pediatric Rheumatology for the purpose of developing in-training, certification, and maintenance of certification examinations. The outline defines the body of knowledge from which the Subboard samples to prepare its examinations. The content specification statements located under each category of the outline are used by item writers to develop questions for the examinations; they broadly address the specific elements of knowledge within each section of the outline. Pediatric Rheumatology Each Pediatric Rheumatology exam is built to the same specifications, also known as the blueprint. This blueprint is used to ensure that, for the initial certification and in-training exams, each exam measures the same depth and breadth of content knowledge. Similarly, the blueprint ensures that the same is true for each Maintenance of Certification exam form. The table below shows the percentage of questions from each of the content domains that will appear on an exam. Please note that the percentages are approximate; actual content may vary. Initial Maintenance of Content Domains Certification Certification Exam Exam 1. Core Knowledge in Scholarly Activities 5% 4% 2. Etiology and Pathophysiology 8% 7% 3. Drug Therapy 10% 12% 4. Musculoskeletal Pain 4% 4% 5. Juvenile Arthritis 18% 18% 6. SLE and Related Disorders 12% 12% 7. Idiopathic Inflammatory Myositis 6.5% 6.5% 8. Vasculitis 7% 7% 9. Sclerodermas and Related Disorders 5% 5% 10. Autoinflammatory Diseases 5% 5% 11. Primary Immunodeficiencies and Other 2% 2% Disorders Associated With Inflammatory and Autoimmune Manifestations 12.
    [Show full text]
  • The Role of the Three Phase Bone Scintigraphy in the Management Of
    Original Article Molecular Imaging and Radionuclide Therapy 2013;22(3): 36-41 DOI: 10.4274/Mirt.68077 The Role of the Three Phase Bone Scintigraphy in the Management of the Patients with Costochondral Pain Kostokondral Ağrısı Olan Hastaların Yönetiminde Üç Fazlı Kemik Sintigrafisinin Rolü Zehra Pınar Koç1, Tansel Ansal Balcı1, M. Oğuzhan Özyurtkan2 1Department of Nuclear Medicine, Firat University, Medical Faculty, Elazığ, Turkey 2Department of Thoracic Surgery, Firat University, Medical Faculty, Elazığ, Turkey Abstract Aim: The bone scintigraphy is indicated in patients with costochondral pain in order to identify the organic etiology. We aimed to investigate the local and projecting pain, or incidental findings in the three phase bone scintigraphy of the patients referred for cos- tochondral pain. Methods: We included 50 patients (36F, 24M; mean: 41±18 years-old) referred to our department for three phase bone scintigraphy for costochondral pain between January 2009-July 2012. Results: Among the 50 patients 22 had normal scintigraphy. An increased activity accumulation in the sternoclavicular joint was observed in 12 patients (right in 4, left in 4 and bilateral in 4) only in late phase and in 9 patients (right in 2, left in 1 and bilateral in 6) with increased vascularity. Among projecting pain causes, activity was present on sternum in 4 patients, on humerus in 2 patients andon the first costa in 2 patients. For the characterization of inflammatory pathology, the three phase bone scintigraphy showed sensitivity, specificity, accuracy, positive and negative predictive values of 43%, 94%, 78%, 77% and 78% respectively. Conclusion: Bone scintigraphy is an effective diagnostic method for the identification of local or projecting pain, and additionally un- expected incidental pathologies associated with costochondral pain.
    [Show full text]
  • Red-Eared Zebra Diagnosis
    CASE REPORT Red-eared zebra diagnosis Editor’s key points Case of relapsing polychondritis Relapsing polychondritis is a rare autoimmune disease that can cause disfgurement and is associated Karen K. Leung MD MSc CCFP Shakibeh Edani MBChB CCFP with potentially life-threatening complications including airway collapse, congestive heart failure, elapsing polychondritis is an autoimmune disease that causes infam- and increased risk of malignancies. mation and destruction of the type 2 collagen found in cartilaginous structures including the pinna, nose, tracheobronchial airways, and Infamed cartilaginous structures Rcardiac valves.1 Both sexes are affected equally,2 with the peak incidence are the most common presentation of relapsing polychondritis, which occurring between the fourth and sixth decades of life.3 Although uncommon, often mimics other conditions with a prevalence of 3.5 to 4.5 cases per million,3 family physicians might be more frequently seen in family the frst professionals to encounter these symptoms, link patients with the medicine such as cellulitis, appropriate specialist services, and make a timely diagnosis. dermatitis, and trauma. Rare diagnoses can also masquerade as common diseases. Upward of 89% of individuals with relapsing polychondritis will develop auricular infamma- Collaboration among medical specialties is important for tion with erythema, swelling, and tenderness,4 which can resemble cellulitis obtaining the diagnosis and or infectious perichondritis,5 as we initially thought in this case. On average,
    [Show full text]